Evolution of height and bone age in primary congenital hypothyroidism.

Thyroid hormones are fundamental for growth and bone maturity. Retarded physical and osseous development signals congenital hypothyroidism. This study assessed the evolution of height and bone age and final height after hormone replacement treatment in 25 patients with primary congenital hypothyroidism. Bone ages, measured periodically for 12 years after treatment began, were expressed as standard deviation scores (SDS) corresponding to chronologic age. Heights were expressed as SDS and related to standardized curves and genetic height. All patients experienced height recovery during the first year. Of 19 patients who reached their final height, 16 surpassed the expected mean for genetic height. Bone age accelerated progressively, with total recovery toward the third year, and remained accelerated, reaching +1.43 +/- 1.27 in relation to chronologic age 12 years after hormone replacement began. Thus, early diagnosis and adequate treatment of congenital hypothyroidism improved growth and osseous development, although progressive acceleration of bone age may have limited final height in some children.

[Inhibition of growth hormone in insulin-dependent diabetics after oral administration of pirenzepine. Fluorophotometric study of the vitreous body]. / Inhibition de l'hormone de croissance chez les sujets diabétiques insulino-dépendants après administration par voie orale de pirenzépine. Etude par fluorophotométrie du vitré.

The purpose of our study was to quantify the blood-retinal barrier permeability in five patients with insulin-dependent diabetic (mean age 14.96 +/- 2.45 years) without retinopathy, after six month with oral pirenzepine, a growth hormone inhibitor. Blood-retinal barrier permeability was determined by vitreous fluorophotometry before and after treatment with a nocturnal oral dose of pirenzepine (0.6 mg/kg the first month and 1 mg/kg the following five months). We found a significant diminution (p < 0.05) in growth hormone levels (10.48 +/- 4.94 ng/ml before treatment and 4.35 +/- 2.53 ng/ml after treatment), nevertheless, no changes in the blood-retinal barrier permeability values were observed (4.84 +/- 2.08 x 10(-6) min-1 before treatment and 4.53 +/- 2.54 x 10(-6) min-1 after treatment). We found no modifications in the levels of somatomedin C or HbA1c either. It can be concluded that oral pirenzepine used at this dose for six months, in spite of a significant decrease in growth hormone secretion, does not decrease the permeability of the blood-retinal barrier.

[Diabetes mellitus]. / Diabetes mellitus.

Some of the most outstanding features of Juvenile Diabetes Mellitus in adolescence are revised in this paper and have been established in a group of 38 affected children controlled in the Department in the past years. The relationship between determined epidemiologic and etiopathogenic factors, as the age in which the disease appears, the sex or the presence of concrete HLA antigens with the clinical expression, evolution and prognosis of the Diabetes Mellitus is underlined. Likewise, some problems about acceptance of the disease, fulfillment of the diet or the self-control that are usually manifested during adolescence are commented.

An 18-month vitreous fluorophotometric follow-up study in juvenile diabetics.

The authors assessed a fluorophotometry follow-up study of 18 months in 24 juvenile insulin-dependent diabetic patients with no retinopathy. The initial duration of diabetes was 5.96 +/- 3.44 years and the glycosylated hemoglobin (HbA1c) was 10.12 +/- 2.27%. The baseline Vitreous Penetration Ratio transmittance value (VPRt) was 4.13 +/- 1.31 x 10-6 min-1 and after 18 months was 5.36 +/- 1.85 x 10-6 min-1, yielding a statistically significant difference (p < 0.01). The average HbA1c during the follow-up term was 9.80 +/- 1.72%. VPRt values were significantly correlated with the duration of diabetes and HbA1c. During the follow-up 3 patients developed foveal background diabetic retinopathy with respect to a high initial VPRt value (6.22 +/- 0.27 x 10-6 min-1), nevertheless, the other 7 patients with high baseline VPRt did not do so. We suggest that vitreous fluorophotometry could be a beneficial procedure in the management of insulin-dependent diabetic patients; however, there is not a cut-off value for VPRt values to help distinguish patients prone to develop retinopathy.

3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: report of five new patients.

Five new patients are reported and the pathogenesis of the hypoglycaemia without ketogenesis is discussed. This report extends a recent review.